Thrombocytopenia can be classified as quantitative (reduced platelet count) or qualitative (platelet function defects). Idiopathic thrombocytopenic purpura (ITP) is an immune-mediated disorder causing reduced platelet counts. It typically presents with purpura and petechiae. Diagnosis involves isolated thrombocytopenia with normal or increased megakaryocytes on bone marrow exam and platelet autoantibodies. Treatment involves corticosteroids, splenectomy, or IVIG. Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy with a classic pentad of signs: microangiopathic hemolytic anemia, thromb

1. BLEEDING
DISORDERS
Dr VIJAY SHANKAR . S.

2. Learning Objectives
 Introduction
 Thromocytopenia – Classification
 Idiopathic thrombocytopenic Purpura
 Thrombotic thrombocytopenic purpura

3. BLEEDING DISORDERS
 Group of disorders characterised by defective
hemostasis with abnormal bleeding
Hemo – Blood
Stasis – Standing still
Spontaneous
Petechiae
Purpura
Ecchymosis
Following trivial trauma

4. Petechiae
( 1 – 2 mm )
Purpura
(≥ 3 mm)
Ecchymosis
(> 1 – 2 cms)

5. Systems Involved in Hemostasis
 Vascular system
 Injured vessel initiates vasoconstriction
 Platelet System
 Injured vessel exposes collagen that initiates platelet
aggregation and help form plug
 Coagulation System
 protein factors of intrinsic and extrinsic pathways produce a
permanent fibrin plug

6. Disorders of Hemostasis
 Vascular disorders:
 Scurvy, easy bruising, Henoch-Schonlein purpura.
 Platelet disorders:
 Quantitative - Thrombocytopenia
 Qualitative - Platelet function disorders – Glanzmans
 Coagulation disorders:
 Congenital - Haemophilia (A, B), Von-Willebrands
 Acquired - Vitamin-K deficiency, Liver disease
 Mixed/Consumption: DIC

7. PLATELET( Thrombocyte)
 Small discoid( 1 – 4 µm) nonnucleate structures
containing granules
 Normal count – 1.5 – 4 lakhs
 Life span – 7 – 8 days

8. Gp IIb/IIIa Complex
(Aggregation)
Gp Ib/IX Complex
(Adhesion)
Platelet ultra structure

9. Investigations of platelets and
platelet functions
Screening tests
1. Platelet count
2. Bleeding time
3. Peripheral smear
Special tests
1. Adhesion tests
2. Aggregation tests
3. Assessment of grannular
content, etc

10. Hemorrhagic diathesis due to
platelet disorders
QUANTITATIVE
↓ in platelet number
( Thrombocytopenia)
QUALITATIVE
Defective platelet function
Congenital Acquired
1. Aspirin therapy
2. Uremia
3. Multiple myeloma, etc
Adhesion defect
Bernard Soulier Synd
VWD
Aggregation defect
Glanzmanns
thrombasthenia

11. THROMBOCYTOPENIAS
 Reduction in the platelet number
 Count below 100,000/µl is generally considered
to constitute thrombocytopenia
 count<20,000/µl-Spontaneous bleeding
 count20,000 – 50,000/µl – aggrevates post
traumatic bleeding

12. • aplasia
• infiltration
• ineffective
megakaryopoiesis
eg. MDS
• selective impairment
of platelet production
•Drugs
• immune
auto-immune (ITP, SLE
drugs
infections
allo-immune
• non-immune
sepsis
DIC, TTP, HUS
hypertensive disorders of
pregnancy
splenomegaly
THROMBOCYTOPENIA
rule out pseudothrombocytopenia
 PRODUCTION SEQUESTRATION↑ DESTRUCTION DILUTIONAL

13. Idiopathic
Thrombocytopenic
Purpura (ITP)

14. Definition
1.Purpura
2.Thrombocytopenia
-Thrombocytes or Platelet
-Penia or Low
3.Idiopathic & Immune

15. Incidence
1. 1 / 10,000 Population
2.Children (age < 15 yr.) 50%
Girl : Boy = 1 : 1
Mortality 0.5 - 1.5%
3.Adults (age 20-40 yr.) 50%
Female : Male = 3-4 : 1
Mortality rare

16. Etiology
.ITP is a disease of increased peripheral
platelet destruction.
.Most patients produce auto-antibodies
to specific platelet membrane
glycoproteins.
.Most patients have either normal or
increased platelet production in BM.

17. Clinical Manifestations
1.Purpura
-Petechiae
-Ecchymoses
2.Hemorrhage

18. Clinical Appearance
1.Acute ITP (children)
2.Chronic ITP (adults)

19. Classification
Mostly children
Male/Female = 1:1
Acute onset
Plt. Count mostly
<20,000/mm3
Spontaneous
remission frequent
Mortality : 0.5-1.5 %
Mostly adults
Male/Female = 1:3-4
Usaully gradual onset
Plt. Count 20,000 –
50,000/mm3
Spontaneous remission rare
Chronic recurrent course
Acute ITP Chronic ITP

20. Common Signs and Symptoms
1.Purpura
2.Menorrhagia
3.Epitaxis
4.Gingival bleeding
5.Recent virus immunization (acute ITP)
6.Recent viral illness (acute ITP)
7.Bruising tendency

21. Role of Spleen
1.Auto-antibody production
2.Platelet destruction
3.Platelet storage

22. Common Physical Findings
Nonpalpable petechiae
Hemorrhage
Purpura
Gingival bleeding
Signs of GI bleeding
Spontaneous bleeding
( plt. < 10,000 /mm3)
Menorrhagia
Retinal hemorrhage
Evidence of intracranial
hemorrhage
Nonpalpable spleen

23. Mortality/Morbidity
1.Hemorrhage represents the most serious
complication
2.Mortality rate from hemorrhage is
approximately 1% in children and 5% in adult
3.Increase risk of severe bleeding in adult ITP
4.Spontaneous remission
: occure in more than 80 % in children
: uncommon in adults

24. Laboratory Examination
1.Complete Blood Cell Count (CBC)
-Isolated thrombocytopenia
2. Increased Bleeding time
3.Bone Marrow Examination
-Megakaryocyte, Megakaryoblast &
Promegakaryocyte -->
increase/normal
-Other cellular component--> normal
4.Platelet Auto-antibody
-PAIgG (non-specific)
-GP specific antibody

25. Fewer Platelets than normal.

26. Two mature megakaryocytes; one with a very high N/C
ratio, the other with a very low N/C ratio.

27. Two bare megakaryocyte nuclear masses

28. Laboratory Findings
1.Isolated thrombocytopenia
2.No splenomegaly
3.Increase megakaryocytes in BM
4.No other cause of thrombocytopenia
5.Platelet auto-antibody found

29. Treatment & Prognosis
Acute ITP
1.Self remission 80 %
2.Platelet transfusion in severe bleeding
3.Corticosteroid therapy within 3-4 weeks
4.No response to corticosteroid > 6 months (15 %)
consider Splenectomy
Chronic ITP
1.Complete remission (10-20 %)
2.Corticosteroid therapy to reduce phagocytic activity of
RE system & suppress antibody production
3.Consider Splenectomy :
-No response to high dose steroid
-Cerebral hemorrhage

30. THROMBOTIC THROMBOCYTOPENIC
PURPURA( TTP)
 Fulminant and lethal disorder
 Characterised by the formation of hyaline
microthrombi within the microvasculature
throughout the body
 The thrombi is composed of platelets and fibrin.

32. Thrombotic Thrombocytopenic
Purpura
 A classic pentad of signs:
 Microangiopathic hemolytic anemia
 Thrombocytopenia
 Neurologic dysfunction
 Renal failure
 Fever
 Untreated, mortality >90%
 Treated with plasmapheresis, mortality <20%
 Incidence ≈4/million/year
 Often strikes young adults, mainly females
 A classic pentad of signs:
 Microangiopathic hemolytic anemia
 Thrombocytopenia
 Neurologic dysfunction
 Renal failure
 Fever

33. PATHOGENISIS
 Defieciency of enzyme – ADAMTS 13
( vWF Metalloproteinase)
Accumulation of
high mol wt multimers of vWF
Promote platelet microaggregate
formation throughout the microcirculation

34.  Untreated, mortality >90%
 Treated with plasmapheresis, mortality <20%

35. Summary
 Thromocytopenia – Classification
 Idiopathic thrombocytopenic Purpura
 Thrombotic thrombocytopenic purpura